Rheumatic fever

Rheumatic fever is a multisystem disorder, occurring as a sequelae to pharyngitis by group A β hemolytic Streptococcus.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 228

Aschoff nodules are composed of multinucleated giant cells surrounded by macrophages and T lymphocytes, and are not seen until the subacute or chronic phases of rheumatic carditis.

They are pathognomonic of RF and occur only in the heart.

Note:

It occurs throughout the heart and is common in the interstitial tissue close to the small blood vessels situated beneath the endocardium of the left ventricle.

* Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 614; Long Cases in Clinical Medicine, ABM Abdullah Page: 228

Subcutaneous nodules are small (0.5–2.0 cm), firm and painless nodules, best felt over extensor surfaces of bone or tendons.

• They occur in 5–7% of patients.
• They typically appear more than 3 weeks after the onset of other manifestations and therefore help to confirm rather than make the diagnosis.

* Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 615

It is a transient, geographical type rash with pink or red raised edges, round margin and clear center.¹

• The lesions start as red macules that fade in the centre but remain red at the edges.
• They occur mainly on the trunk and proximal extremities but not the face.
• The resulting red rings or ‘margins’ may coalesce or overlap.
• Erythema marginatum occurs in less than 5% of patients.

* Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 615; ¹Long Cases in Clinical Medicine, ABM Abdullah Page: 228

This is a late neurological manifestation that appears at least 3 months after the episode of acute rheumatic fever, when all the other signs may have disappeared.

• It occurs in up to one-third of cases and is more common in females.
• Emotional lability may be the first feature and is typically followed by purposeless, involuntary, choreiform movements of the hands, feet or face.
• Speech may be explosive and halting.
• Spontaneous recovery usually occurs within a few months.
• Approximately one-quarter of affected patients will go on to develop chronic rheumatic valve disease.

Note:¹

• Relapse may occur only in few cases, occasionally during pregnancy (called chorea gravidarum) or in those who use oral contraceptive pill.

* Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 615; ¹Long Cases in Clinical Medicine, ABM Abdullah Page: 229

Children between 5-15 years of age have high risk of streptococcal pharyngitis and acute rheumatic fever.

* Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 137, Pre-exam preparation for medicine, HN Sarker

The condition is triggered by an immune-mediated delayed response to infection with specific strains of group A β-hemolytic streptococci due to antigenic molecular mimicry.

• Autoimmune reaction between the antigen (M protein) of Streptococcus β hemolyticus and cardiac myosin and sarcolemal membrane protein (laminin).
• Antibody is produced against streptococcal enzyme, causing inflammation in the endocardium, myocardium and pericardium as well as joints and skin.

* Pre-exam preparation for medicine, HN Sarker; Long Cases in Clinical Medicine, ABM Abdullah Page: 227; Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 141

• Acute rheumatic fever is a multisystem disorder that usually presents with fever, anorexia, lethargy and joint pain, 2–3 weeks after an episode of streptococcal pharyngitis. There may, however, be no history of sore throat.
• Arthritis occurs in approximately 75% of patients.
• Other features include rashes, carditis (e.g. palpitation and chest pain) and neurological changes (e.g. involuntary movement – chorea).

Figure: Clinical features of rheumatic fever

* Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 614; Long Cases in Clinical Medicine, ABM Abdullah Page: 227

Large joints typically affects the –

1. Knees,
2. Ankles,
3. Elbows and
4. Wrists

Note:

RF usually does not involve small joints of the hands and feet.

* Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 615

Migratory polyarthritis.

* Pre-exam preparation for medicine, HN Sarker

As follows:

• Rheumatic fever
• Septicemia
• Gonococcal arthritis
• Syphilitic arthritis
• Lyme arthritis
• Hyperlipidemia (type 2)

* Long Cases in Clinical Medicine, ABM Abdullah Page: 230

Arthralgia means subjective joint pain but no swelling. Arthritis means joint pain with swelling.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 230

Characteristic movements

• Milkmaid grip
• Spooning and pronation of hands when patient’s arms are extended
• Wormian / darting movements of the tongue upon protrusion
• Deterioration of handwriting

Associated features

• Emotional lability, incoordination, poor school performance and facial grimacing. These are exacerbated by stress and disappears with sleep.

Figure: Sydenham’s chorea

* Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 139

Rheumatic fever can cause carditis involving all the layers of the heart (endocardium, myocardium and pericardium), called pancarditis.

Symptoms of carditis:

• It may manifest as breathlessness (due to heart failure or pericardial effusion), palpitations or chest pain (usually due to pericarditis or pancarditis).
• Pericarditis may cause chest pain and precordial tenderness.
• Cardiac failure may be due to myocardial dysfunction or valvular regurgitation.
• Conduction defects sometimes occur and may cause syncope.

* Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 614

As follows:

• Persistent fever
• Tachycardia
• High ESR
• Leukocytosis
• Evidence of carditis.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 229

Rheumatic fever can cause carditis involving all the layers of the heart (endocardium, myocardium and pericardium), called pancarditis.

Signs of endocarditis:

• Soft heart sounds
• A soft systolic murmur due to mitral regurgitation is very common
• Pansystolic murmur (due to MR)
• Early diastolic murmur (due to AR which is due to valvulitis with nodules on the valve).
• A soft mid-diastolic murmur (the Carey Coombs murmur) is typically due to valvulitis, with nodules forming on the mitral valve leaflets.

Signs of myocarditis:

• Tachycardia
• Conduction defect
• Soft heart sounds, S3 gallop
• Cardiomegaly
• Features of heart failure.

Signs of pericarditis:

• Pericardial rub (patient usually complains of chest pain)
• Pericardial effusion may be present.

* Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 614; Long Cases in Clinical Medicine, ABM Abdullah Page: 228; Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 138

Investigations in acute rheumatic fever –

Evidence of a systemic illness (non-specific)

• Complete blood count: Hb% (normal) TC, DC (Leucocytosis),
• Acute phase reactants (ESR, CRP): Raised ESR and CRP

Evidence of preceding streptococcal infection (specific)

• Throat swab culture: Group A β-haemolytic streptococci (also from family members and contacts)
• Antistreptolysin O antibodies (ASO titres): rising titres, or levels of > 200 U (adults) or > 300 U (children)

Evidence of carditis

• Chest X-ray: Cardiomegaly; pulmonary congestion
• ECG: First-degree AV block (prolonged P-R interval) and rarely second-degree AV block; features of pericarditis; T-wave inversion; reduction in QRS voltages
• Echocardiography: Cardiac dilatation and valve abnormalities

Others (if needed)

• RA factor (to exclude rheumatoid arthritis) and ANA (to exclude SLE).

* Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 615; Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 139; Long Cases in Clinical Medicine, ABM Abdullah Page: 226

The diagnosis, made using the revised Jones criteria.

Diagnosis: Two or more major manifestations, or one major and two or more minor manifestations, along with evidence of preceding streptococcal infection.

Figure: Diagnostic criteria of rheumatic fever

* Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 614

As follows:

• Infective arthritis (viral or bacterial)
• Juvenile chronic arthritis (juvenile <16 years)
• SLE
• Seronegative arthritis (reactive arthritis, Reiter’s syndrome)
• Rheumatoid arthritis.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 226

This young patient has a history of sore throat followed by a latent period and then she developed migrating, inflammatory polyarthritis involving the large joints without any deformity. All these are in favor of acute rheumatic fever.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 226

Because, in this case, arthritis is fleeting is nature involving the bigger joints that is not common in SLE. Also, other criteria of SLE like skin rash, butterfly rash, mouth ulcer, alopecia, menstrual irregularity, etc. are absent.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 226

JIA occurs in young patient less than 16 years of age. The arthritis is not fleeting in nature. Also, in JIA there is usually deformity of the joints.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 226

Reiter’s syndrome is characterized by triad of iritis, arthritis and urethritis. But the patient does not have history of involvement of the eyes or urinary tract. Also, it occurs after an episode of diarrhea or urethritis which are not present here.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 226

Acute rheumatic fever.

Acute rheumatic fever with heart failure.

Management of the acute attack

1. Counsel the patents about the disease, its complications and importance of strict adherence to penicillin prophylaxis.
2. Supportive
   • Bed rest (until disease activity resolvese e.g. temperature, leukocyte count, and ESR become normal).
   • Immobilization of the affected joints
   • Close monitoring for features of carditis
3. Antibiotic therapy: to eliminate residual streptococcal infection.
   • A single IM injection of benzathine penicillin
     • Dose: <30 kg – 6 lacs unit
     • >30 kg – 12 lacs unit (1.2 million U)
   • Or, phenoxymethylpenicillin
     • Dose: 250 mg 6-hourly for 10 days (50 mg/kg/day in children) .
   • Or, if the patient is penicillin-allergic, erythromycin or a cephalosporin.
4. Anti-inflammatory drugs:
   • Aspirin
     • Indications: patient with
       • polyartheritis,
       • isolated carditis without cardiolmegaly or CCF
     • Dose:
       • 60 mg/kg/day, divided into 6 doses.
       • In adults, 100 mg/kg/day may be needed upto the limits of tolerance or a maximum of 8 gm/day.
       • Continue until the ESR has fallen and then gradually tailed off.
   • Prednisone
     • Indications: Patient with
       • Severe arthritis
       • Carditis and cardiomegaly or CCF
     • Dose
       • 1.0–2.0 mg/kg/day in 4 divided doses
       • Continue until the ESR is normal, then tailed off.

Note

When prednisone is being tapered,aspirin should be started at 50 mg/kg/day in 4 divided doses for 6 weeks to prevent rebound inflammation.

* Davidson’s Principles and Practice of Medicine, 22nd Edition Page: 615; Pre-exam preparation for medicine, HN Sarker; Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 139, 140

Both initial and subsequent attacks of ARF can be prevented through penicillin prophylaxis.

Prevention of initial attack (Primary prevention):

• Phenoxymethylpenicillin or erythromycin orally for 10 days in any case of streptococcal sore throat.

Prevention of subsequent attack (Secondary prevention):

• Benzathine penicillin injection IM  monthly. Dose –
  • <30 kg – 6 lacs unit
  • >30 kg – 12 lacs unit (1.2 million U)
• Or, oral phenoxymethylpenicillin (Penicillin V). Dose –
  • 250 mg 12-hourly (weight >30 kg)
  • 150 mg 12-hourly (weight <30 kg)

Duration of penicillin prophylaxis:

* Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 140, 141

Treatment of Sydenham’s chorea:

• Phenobarbital (16-32 mg every 6-8 hour PO) is the drug of choice
• If phenobarbital is ineffective, either of the following should be initiated–
  • Haloperidol (0.01-0.03 mg/kg/24 hour PO in 2 divided doses)
  • Chlorpromazine (0. 5 mg/kg every 4-6 hourly PO)
• Antiinflammatory agents are usually not indicated
• Duration of treatment : Depends on the response.
• Dose is increased until desired response is achieved then tapered gradually.

* Step on to Paediatrics, Md Abid Hossain Mollah, 3rd Edition Page: 140

• Acute attack may last up to 3 months, but recurrence may be precipitated by streptococcal infections, pregnancy, use of oral contraceptive pill, etc. 60% patients with carditis develop chronic rheumatic heat disease.
• Mitral valve is most commonly involved followed by aortic valve.
• Tricuspid and pulmonary valves are rarely involved.
• Usually, regurgitation of cardiac valves develops during acute attacks, while stenosis develops years later.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 229

• Skin infection with streptococci is not associated with RF. It may be associated with acute post streptococcal glomerulonephritis.
• 2 to 3% of previously healthy person may suffer from rheumatic fever following streptococcal pharyngitis.
• Streptococcal sore throat may not be present in some cases.
• More than 50% patients of RF with carditis will develop chronic valvular disease after 10 to 20 years. All the cardiac valves may be involved, but most commonly the mitral valve is affected (90%). Also aortic valve may be involved. Involvement of the tricuspid and pulmonary valves is rare.
• Arthritis in rheumatic fever recovers completely without any residual change (Rheumatic fever licks the joints and kills the heart). However, a rare type of arthritis called Jaccoud’s arthritis is associated with deformity of metacarpophalangeal joints after repeated attack of rheumatic fever.

* Long Cases in Clinical Medicine, ABM Abdullah Page: 230

Q. What are the rheumatic heart diseases?

• Acute RF
• Chronic rheumatic heart disease

Q. How many days are required for development of RF and why?

RF requires 2-3 weeks to develop due to formation of antibody.

Q. What type of hypersensitivity acute RF is?

Type-II hypersensitivity.